Department of Medical Sciences and Public Health, Institute of Neurology, University of Cagliari and AOU Cagliari, Cagliari, 09042, Italy.
Department of Medical Sciences and Public Health, Institute of Neurology, University of Cagliari and AOU Cagliari, Cagliari, 09042, Italy.
Seizure. 2019 Aug;70:1-5. doi: 10.1016/j.seizure.2019.06.020. Epub 2019 Jun 13.
Hashimoto's encephalopathy is a non-infectious, probably autoimmune encephalitis, characterized by varied signs coupled with elevated levels of anti-thyroid antibodies and, often, good response to corticosteroid therapy. Seizures, namely focal and generalized tonic-clonic seizures, myoclonus, and status epilepticus, are frequent manifestations of Hashimoto's encephalopathy. Typically, seizures in these patients respond poorly to anti-epileptic drugs. Although cases of Hashimoto's encephalopathy with status epilepticus have been reported in literature, they vary in demographic, clinical, and treatment characteristics. We could not identify any systematic review summarizing the evidence in regard to factors predicting the occurrence of status epilepticus in Hashimoto's encephalopathy and the responsiveness of status epilepticus to anti-epileptic drugs, steroids and other immunomodulatory medication. Therefore, we performed an extensive review of the literature to identify and compare Hashimoto's encephalopathy patients presenting with and without status epilepticus. In 31 patients with status epilepticus and 104 patients without status epilepticus, thyroid status, anti-thyroid antibodies, cerebrospinal fluid analysis, brain MRI/CT/SPECT scan did not predict occurrence of status epilepticus of variable phenomenology. Status epilepticus did not respond to anti-epileptic drugs but completely remitted under steroid treatment, alone or in combination with other immunomodulatory medication, in about three quarter of patients. Generalized convulsive status epilepticus might be a factor negatively influencing outcome.
桥本脑病是一种非传染性、可能自身免疫性的脑炎，其特征是多种表现并存，同时伴有甲状腺抗体水平升高，且通常对皮质类固醇治疗反应良好。癫痫发作，即局灶性和全面性强直阵挛发作、肌阵挛和癫痫持续状态，是桥本脑病的常见表现。这些患者的癫痫发作通常对抗癫痫药物反应不佳。尽管文献中已经报道了伴有癫痫持续状态的桥本脑病病例，但它们在人口统计学、临床和治疗特征方面存在差异。我们无法确定任何系统评价来总结有关预测桥本脑病中癫痫持续状态发生的因素以及癫痫持续状态对抗癫痫药物、类固醇和其他免疫调节药物反应的证据。因此，我们对文献进行了广泛的回顾，以确定并比较出现和未出现癫痫持续状态的桥本脑病患者。在 31 例伴有癫痫持续状态的患者和 104 例无癫痫持续状态的患者中，甲状腺状态、甲状腺抗体、脑脊液分析、脑 MRI/CT/SPECT 扫描均不能预测具有不同表现的癫痫持续状态的发生。癫痫持续状态对抗癫痫药物无反应，但在皮质类固醇单独或联合其他免疫调节药物治疗下，约四分之三的患者完全缓解。全身性强直阵挛性癫痫持续状态可能是影响预后的一个负面因素。
